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Items: 20

1.

Huntingtin suppression restores cognitive function in a mouse model of Huntington's disease.

Southwell AL, Kordasiewicz HB, Langbehn D, Skotte NH, Parsons MP, Villanueva EB, Caron NS, Østergaard ME, Anderson LM, Xie Y, Cengio LD, Findlay-Black H, Doty CN, Fitsimmons B, Swayze EE, Seth PP, Raymond LA, Frank Bennett C, Hayden MR.

Sci Transl Med. 2018 Oct 3;10(461). pii: eaar3959. doi: 10.1126/scitranslmed.aar3959.

PMID:
30282695
2.

A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles.

Southwell AL, Skotte NH, Villanueva EB, Østergaard ME, Gu X, Kordasiewicz HB, Kay C, Cheung D, Xie Y, Waltl S, Dal Cengio L, Findlay-Black H, Doty CN, Petoukhov E, Iworima D, Slama R, Ooi J, Pouladi MA, Yang XW, Swayze EE, Seth PP, Hayden MR.

Hum Mol Genet. 2017 Mar 15;26(6):1115-1132. doi: 10.1093/hmg/ddx021.

PMID:
28104789
3.

Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry.

Kay C, Collins JA, Skotte NH, Southwell AL, Warby SC, Caron NS, Doty CN, Nguyen B, Griguoli A, Ross CJ, Squitieri F, Hayden MR.

Mol Ther. 2015 Nov;23(11):1759-1771. doi: 10.1038/mt.2015.128. Epub 2015 Jul 23.

4.

A SNP in the HTT promoter alters NF-κB binding and is a bidirectional genetic modifier of Huntington disease.

Bečanović K, Nørremølle A, Neal SJ, Kay C, Collins JA, Arenillas D, Lilja T, Gaudenzi G, Manoharan S, Doty CN, Beck J, Lahiri N, Portales-Casamar E, Warby SC, Connolly C, De Souza RA; REGISTRY Investigators of the European Huntington's Disease Network, Tabrizi SJ, Hermanson O, Langbehn DR, Hayden MR, Wasserman WW, Leavitt BR.

Nat Neurosci. 2015 Jun;18(6):807-16. doi: 10.1038/nn.4014. Epub 2015 May 4.

PMID:
25938884
5.

HD iPSC-derived neural progenitors accumulate in culture and are susceptible to BDNF withdrawal due to glutamate toxicity.

Mattis VB, Tom C, Akimov S, Saeedian J, Østergaard ME, Southwell AL, Doty CN, Ornelas L, Sahabian A, Lenaeus L, Mandefro B, Sareen D, Arjomand J, Hayden MR, Ross CA, Svendsen CN.

Hum Mol Genet. 2015 Jun 1;24(11):3257-71. doi: 10.1093/hmg/ddv080. Epub 2015 Mar 3.

6.

Allele-specific suppression of mutant huntingtin using antisense oligonucleotides: providing a therapeutic option for all Huntington disease patients.

Skotte NH, Southwell AL, Østergaard ME, Carroll JB, Warby SC, Doty CN, Petoukhov E, Vaid K, Kordasiewicz H, Watt AT, Freier SM, Hung G, Seth PP, Bennett CF, Swayze EE, Hayden MR.

PLoS One. 2014 Sep 10;9(9):e107434. doi: 10.1371/journal.pone.0107434. eCollection 2014.

7.

In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides.

Southwell AL, Skotte NH, Kordasiewicz HB, Østergaard ME, Watt AT, Carroll JB, Doty CN, Villanueva EB, Petoukhov E, Vaid K, Xie Y, Freier SM, Swayze EE, Seth PP, Bennett CF, Hayden MR.

Mol Ther. 2014 Dec;22(12):2093-106. doi: 10.1038/mt.2014.153. Epub 2014 Aug 7.

8.

Bidirectional control of postsynaptic density-95 (PSD-95) clustering by Huntingtin.

Parsons MP, Kang R, Buren C, Dau A, Southwell AL, Doty CN, Sanders SS, Hayden MR, Raymond LA.

J Biol Chem. 2014 Feb 7;289(6):3518-28. doi: 10.1074/jbc.M113.513945. Epub 2013 Dec 17.

9.

High frequency of intermediate alleles on Huntington disease-associated haplotypes in British Columbia's general population.

Semaka A, Kay C, Doty CN, Collins JA, Tam N, Hayden MR.

Am J Med Genet B Neuropsychiatr Genet. 2013 Dec;162B(8):864-71. doi: 10.1002/ajmg.b.32193. Epub 2013 Aug 30.

PMID:
24038799
10.

Rational design of antisense oligonucleotides targeting single nucleotide polymorphisms for potent and allele selective suppression of mutant Huntingtin in the CNS.

Østergaard ME, Southwell AL, Kordasiewicz H, Watt AT, Skotte NH, Doty CN, Vaid K, Villanueva EB, Swayze EE, Bennett CF, Hayden MR, Seth PP.

Nucleic Acids Res. 2013 Nov;41(21):9634-50. doi: 10.1093/nar/gkt725. Epub 2013 Aug 19.

11.

Huntington disease in the South African population occurs on diverse and ethnically distinct genetic haplotypes.

Baine FK, Kay C, Ketelaar ME, Collins JA, Semaka A, Doty CN, Krause A, Greenberg LJ, Hayden MR.

Eur J Hum Genet. 2013 Oct;21(10):1120-7. doi: 10.1038/ejhg.2013.2. Epub 2013 Mar 6.

12.

Hip14l-deficient mice develop neuropathological and behavioural features of Huntington disease.

Sutton LM, Sanders SS, Butland SL, Singaraja RR, Franciosi S, Southwell AL, Doty CN, Schmidt ME, Mui KK, Kovalik V, Young FB, Zhang W, Hayden MR.

Hum Mol Genet. 2013 Feb 1;22(3):452-65. doi: 10.1093/hmg/dds441. Epub 2012 Oct 16.

PMID:
23077216
13.

A fully humanized transgenic mouse model of Huntington disease.

Southwell AL, Warby SC, Carroll JB, Doty CN, Skotte NH, Zhang W, Villanueva EB, Kovalik V, Xie Y, Pouladi MA, Collins JA, Yang XW, Franciosi S, Hayden MR.

Hum Mol Genet. 2013 Jan 1;22(1):18-34. doi: 10.1093/hmg/dds397. Epub 2012 Sep 21.

14.

Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin.

Carroll JB, Warby SC, Southwell AL, Doty CN, Greenlee S, Skotte N, Hung G, Bennett CF, Freier SM, Hayden MR.

Mol Ther. 2011 Dec;19(12):2178-85. doi: 10.1038/mt.2011.201. Epub 2011 Oct 4.

15.

HTT haplotypes contribute to differences in Huntington disease prevalence between Europe and East Asia.

Warby SC, Visscher H, Collins JA, Doty CN, Carter C, Butland SL, Hayden AR, Kanazawa I, Ross CJ, Hayden MR.

Eur J Hum Genet. 2011 May;19(5):561-6. doi: 10.1038/ejhg.2010.229. Epub 2011 Jan 19.

16.

SCN9A: another sodium channel excited to play a role in human epilepsies.

Doty CN.

Clin Genet. 2010 Apr;77(4):326-8. doi: 10.1111/j.1399-0004.2009.01366_1.x. Epub 2010 Jan 20. No abstract available.

PMID:
20095983
17.

Finding bald spots on chromosome 20p11.

Doty CN.

Clin Genet. 2009 Mar;75(3):226-7. doi: 10.1111/j.1399-0004.2009.01150_2.x. No abstract available.

PMID:
19250378
18.

Phosphorylation of huntingtin reduces the accumulation of its nuclear fragments.

Warby SC, Doty CN, Graham RK, Shively J, Singaraja RR, Hayden MR.

Mol Cell Neurosci. 2009 Feb;40(2):121-7. doi: 10.1016/j.mcn.2008.09.007. Epub 2008 Oct 18.

PMID:
18992820
19.

Activated caspase-6 and caspase-6-cleaved fragments of huntingtin specifically colocalize in the nucleus.

Warby SC, Doty CN, Graham RK, Carroll JB, Yang YZ, Singaraja RR, Overall CM, Hayden MR.

Hum Mol Genet. 2008 Aug 1;17(15):2390-404. doi: 10.1093/hmg/ddn139. Epub 2008 Apr 29.

PMID:
18445618
20.

Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin.

Graham RK, Deng Y, Slow EJ, Haigh B, Bissada N, Lu G, Pearson J, Shehadeh J, Bertram L, Murphy Z, Warby SC, Doty CN, Roy S, Wellington CL, Leavitt BR, Raymond LA, Nicholson DW, Hayden MR.

Cell. 2006 Jun 16;125(6):1179-91.

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